Primary sclerosing (skluh-ROHS-ing) cholangitis (koh-lan-JIE-tis) is a disease of the bile ducts. Bile ducts carry the digestive liquid bile from your liver to your small intestine. In primary sclerosing cholangitis, inflammation causes scars within the bile ducts. These scars make the ducts hard and narrow and gradually cause serious liver damage.
In most people with primary sclerosing cholangitis, the disease progresses slowly. It can eventually lead to liver failure, repeated infections, and tumors of the bile duct or liver. A liver transplant is the only known cure for advanced primary sclerosing cholangitis, but the disease may recur in the transplanted liver in a small number of patients.
Care for primary sclerosing cholangitis focuses on monitoring liver function, managing symptoms and, when possible, doing procedures that temporarily open blocked bile ducts.
Primary sclerosing cholangitis care at Mayo Clinic
Source: http://www.mayoclinic.com
Primary sclerosing cholangitis is often diagnosed before symptoms appear when a routine blood test or an X-ray taken for an unrelated condition shows liver abnormalities.
Early symptoms often include:
Many people diagnosed with primary sclerosing cholangitis before they have symptoms continue to feel generally well for several years. But there's no reliable way to predict how quickly or slowly the disease will progress for any individual.
Signs and symptoms that may appear as the disease progresses include:
Make an appointment with your doctor if you have severe, unexplained itching on much of your body â itching that persists no matter how much you scratch. Also see your doctor if you feel extremely tired all the time, no matter what you do.
It's particularly important to bring unexplained fatigue and itching to your doctor's attention if you have ulcerative colitis or Crohn's disease, both of which are types of inflammatory bowel disease. A majority of people with primary sclerosing cholangitis also have one of these diseases.
Source: http://www.mayoclinic.com
It's not clear what causes primary sclerosing cholangitis. An immune system reaction to an infection or toxin may trigger the disease in people who are genetically predisposed to it.
A large proportion of people with primary sclerosing cholangitis also have inflammatory bowel disease, an umbrella term that includes ulcerative colitis and Crohn's disease.
Primary sclerosing cholangitis and inflammatory bowel disease don't always appear at the same time, though. In some cases, primary sclerosing cholangitis is present for years before inflammatory bowel disease occurs. If primary sclerosing cholangitis is diagnosed, it's important to look for inflammatory bowel disease because there is a greater risk of colon cancer.
Somewhat less often, people being treated for inflammatory bowel disease turn out to have primary sclerosing cholangitis as well. And rarely, people with primary sclerosing cholangitis develop inflammatory bowel disease only after having a liver transplant.
Source: http://www.mayoclinic.com
Tests and procedures used to diagnose primary sclerosing cholangitis include:
X-rays of your bile ducts. A type of bile duct X-ray called endoscopic retrograde cholangiopancreatography (ERCP) in addition to, or instead of, an MRI may be needed. But this test is rarely used for diagnosis because of the risk of complications.
To make your bile ducts visible on an X-ray, your doctor uses a flexible tube passed down your throat to inject dye into the area of your small intestine where your bile ducts empty.
An ERCP is the test of choice if signs and symptoms persist despite no abnormalities on an MRI. An ERCP is often the initial test if you're unable to have an MRI because of a metal implant in your body.
Testing a sample of liver tissue. A liver biopsy is a procedure to remove a piece of liver tissue for laboratory testing. Your doctor inserts a needle through your skin and into your liver to extract a tissue sample.
A liver biopsy can help determine the extent of damage to your liver. The test is used only when the diagnosis of primary sclerosing cholangitis is still uncertain after less invasive tests.
Source: http://www.mayoclinic.com
Complications of primary sclerosing cholangitis may include:
Portal hypertension. Your portal vein is the major route for blood flowing from your digestive system into your liver. Portal hypertension refers to high blood pressure in this vein.
Portal hypertension can cause fluid from the liver to leak into your abdominal cavity (ascites). It can also divert blood from the portal vein to other veins, causing these veins to become swollen (varices). Varices are weak veins and tend to bleed easily, which can be life-threatening.
Source: http://www.mayoclinic.com
No alternative medicine treatments have been found to treat primary sclerosing cholangitis. But some complementary and alternative therapies may help you cope with the signs and symptoms of the disease. Talk to your doctor about your options.
Fatigue is common in people with primary sclerosing cholangitis. While doctors can treat some factors that may contribute to fatigue, your signs and symptoms may still persist. You might find relief with complementary and alternative treatments that have shown some benefit for fatigue, such as:
Source: http://www.mayoclinic.com
If you've been diagnosed with primary sclerosing cholangitis, take steps to care for your liver, such as:
Source: http://www.mayoclinic.com
Factors that may increase the risk of primary sclerosing cholangitis include:
Source: http://www.mayoclinic.com
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